Systemic cutaneous anaplastic large t-cell lymphoma



A 53-year-old male with a history of cutaneous anaplastic T- cell lymphoma treated with radiotherapy presented with shiny erythematous noduleson bilateral shoulders. He also reported a new swelling in his right submandibular region. Punch biopsy of a nodule on the shoulder showed atypical lymphoid population in the dermis with only scant extension to the epidermis. There were scant nuclei that appeared binucleated and had a reniform (Hallmark like) appearance. There was CD30 and CD56 positivity (a spectrum of T-cell marker positivity) and ALK negativity. These features were consistent with the known cutaneous anaplastic large T- cell lymphoma (Stage 11-AE), ALK negative. The patient received systemic chemotherapy with CHOP (cyclophosphomide, doxorubicin, vincristine and prednisolone) and was planned for an allogeneic stem cell transplant in the near future.
Anaplastic large cell lymphomas are malignancies of mature T lymphocytes that express the lymphocyte activation marker CD30. It comprises approximately 3% of all adult Non-Hodgkin lymphomas and 10% to 20% of childhood lymphomas. The majority of patients are male and present with advanced stage III to IV disease and often with B symptoms such as fevers, chills and night sweats. The skin, soft tissue, bone and bone marrow are common extranodal sites of involvement. Standard chemotherapy with CHOP is used for aggressive lymphomas, including cutaneous anaplastic large T- cell lymphoma (1).
References
Hapgood G, Savage KJ. The biology and management of systemic anaplastic large cell lymphoma. Blood. 2015 Jul 2;126(1):17-25


Dr. Pooja Kadam